Access the full text.
Sign up today, get DeepDyve free for 14 days.
R. Bubik, Dylan Barth, C. Hook, R. Wolf, Jessica Muth, K. Mara, M. Patnaik, R. Pruthi, A. Marshall, M. Litzow, M. Elliott, W. Hogan, M. Shah, K. Begna, H. Alkhateeb, A. Pardanani, A. Ashrani, T. Call, C. Rivera, J. Camoriano, R. Go, A. Wolanskyj-Spinner, S. Parikh (2020)
Clinical outcomes of adults with hemophagocytic lymphohistiocytosis treated with the HLH-04 protocol: a retrospective analysisLeukemia & Lymphoma, 61
J Li, Q Wang, W Zheng, J Ma, W Zhang, W Wang (2014)
Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients, 93
A. Schram, Paige Comstock, M. Campo, D. Gorovets, A. Mullally, Kelly Bodio, J. Arnason, N. Berliner (2016)
Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 yearsBritish Journal of Haematology, 172
P. Mehta, R. Cron, J. Hartwell, J. Manson, R. Tattersall (2020)
Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndromeThe Lancet Rheumatology, 2
Asra Ahmed, Samuel Merrill, F. Alsawah, P. Bockenstedt, E. Campagnaro, S. Devata, S. Gitlin, M. Kaminski, Alice Cusick, T. Phillips, S. Sood, M. Talpaz, A. Quiery, P. Boonstra, R. Wilcox (2019)
Ruxolitinib in adult patients with secondary haemophagocytic lymphohistiocytosis: an open-label, single-centre, pilot trial.The Lancet. Haematology
S. Parikh, P. Kapoor, L. Letendre, Shaji Kumar, A. Wolanskyj (2014)
Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis.Mayo Clinic proceedings, 89 4
Tiffany Lin, Laura Ferlic-Stark, C. Allen, C. Kozinetz, K. McClain (2011)
Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortalityPediatric Blood & Cancer, 56
L. Naymagon, D. Tremblay, J. Mascarenhas (2020)
Reevaluating the role of ferritin in the diagnosis of adult secondary hemophagocytic lymphohistiocytosisEuropean Journal of Haematology, 104
J. Henter, A. Horne, M. Aricò, R. Egeler, A. Filipovich, S. Imashuku, S. Ladisch, K. McClain, D. Webb, J. Winiarski, G. Janka (2007)
HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosisPediatric Blood & Cancer, 48
Jia Zhang, Yuan Sun, Xiaodong Shi, Rui Zhang, Yini Wang, Juan Xiao, Jing Cao, Zhuo Gao, Jingshi Wang, Lin Wu, Wei Wei, Zhao Wang (2020)
Genotype characteristics and immunological indicator evaluation of 311 hemophagocytic lymphohistiocytosis cases in ChinaOrphanet Journal of Rare Diseases, 15
M. Vallurupalli, N. Berliner (2019)
Emapalumab for the treatment of relapsed/refractory hemophagocytic lymphohistiocytosis.Blood
L. Naymagon, D. Tremblay, K. Troy, J. Mascarenhas (2020)
Soluble Interleukin-2 Receptor (sIL-2r) Level is a Limited Test for the Diagnosis of Adult Secondary Hemophagocytic Lymphohistiocytosis (HLH).European journal of haematology
P. Rosée, A. Horne, M. Hines, Tatiana Greenwood, Rafal Machowicz, N. Berliner, S. Birndt, J. Gil-Herrera, M. Girschikofsky, M. Jordan, Ashish Kumar, J. Laar, G. Lachmann, K. Nichols, A. Ramanan, Yini Wang, Zhao Wang, G. Janka, J. Henter (2019)
Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.Blood, 133 23
Julien Carvelli, C. Pipéroglou, C. Farnarier, F. Vely, K. Mazodier, S. Audonnet, P. Nitschké, C. Bole-Feysot, M. Boucekine, A. Cambon, M. Hamidou, J. Harlé, G. Basile, G. Kaplanski (2020)
FUNCTIONAL AND GENETIC TESTING IN ADULTS WITH HLH DO NOT REVEAL A CYTOTOXICITY DEFECT BUT RATHER AN INFLAMMATORY PROFILE.Blood
Z. Otrock, C. Eby (2015)
Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosisAmerican Journal of Hematology, 90
Etienne Dufranc, A. Bello, J. Belliere, N. Kamar, S. Faguer, Etienne Faguer, Etienne Dufranc, A. Bello, J. Belliere, N. Kamar, S. Faguer (2020)
IL6-R blocking with tocilizumab in critically ill patients with hemophagocytic syndromeCritical Care, 24
G. Tamamyan, H. Kantarjian, J. Ning, P. Jain, K. Sasaki, K. McClain, C. Allen, S. Pierce, J. Cortes, F. Ravandi, M. Konopleva, G. Garcia-Manero, C. Benton, D. Chihara, M. Rytting, Sa Wang, Waleed Abdelall, S. Konoplev, N. Daver (2016)
Malignancy‐associated hemophagocytic lymphohistiocytosis in adults: Relation to hemophagocytosis, characteristics, and outcomesCancer, 122
Elisabet Bergsten, Elisabet Bergsten, A. Horne, A. Horne, M. Aricò, I. Astigarraga, R. Egeler, A. Filipovich, E. Ishii, G. Janka, S. Ladisch, K. Lehmberg, K. McClain, M. Minkov, Scott Montgomery, Scott Montgomery, Scott Montgomery, V. Nanduri, D. Rosso, J. Henter, J. Henter (2017)
Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study.Blood, 130 25
M. Arca, L. Fardet, L. Galicier, S. Rivière, C. Marzac, C. Aumont, O. Lambotte, P. Coppo (2015)
Prognostic factors of early death in a cohort of 162 adult haemophagocytic syndrome: impact of triggering disease and early treatment with etoposideBritish Journal of Haematology, 168
Jun Zhou, Jingjing Zhou, Dan-Ting Shen, H. Goyal, Zhi‐Qi Wu, Hua-Guo Xu (2020)
Development and validation of the prognostic value of ferritin in adult patients with Hemophagocytic LymphohistiocytosisOrphanet Journal of Rare Diseases, 15
E. Draper, D. Wagner, J. Zimmerman (1985)
APACHE II: A severity of disease classification systemCritical Care Medicine, 13
Steven Austin, Y. Wong, R. Uzzo, J. Beck, B. Egleston (2015)
Why Summary Comorbidity Measures Such As the Charlson Comorbidity Index and Elixhauser Score WorkMedical Care, 53
Meng Zhou, Li Li, Q. Zhang, Shanshan Ma, Jianai Sun, Lixia Zhu, Danlei Lu, J. Zhu, Daizhan Zhou, Yanlong Zheng, Xiudi Yang, Mixue Xie, Mingyu Zhu, X. Ye, W. Xie (2018)
Clinical features and outcomes in secondary adult hemophagocytic lymphohistiocytosisQJM: An International Journal of Medicine, 111
M. Zinter, M. Hermiston (2019)
Calming the storm in HLH.Blood, 134 2
Data supporting the use of etoposide-based therapy in hemophagocytic lymphohistiocytosis (HLH) arise largely from pediatric studies. There is a lack of comparable data among adult patients with secondary HLH. We conducted a retrospective study to assess the impact of etoposide-based therapy on outcomes in adult secondary HLH. The primary outcome was overall survival. The log-rank test was used to compare Kaplan-Meier distributions of time-to-event outcomes. Multivariable Cox proportional hazards modeling was used to estimate adjusted hazard ratios (HRs) with 95% confidence intervals (CIs). Ninety adults with secondary HLH seen between January 1, 2009, and January 6, 2020, were included. Forty-two patients (47%) received etoposide-based therapy, while 48 (53%) received treatment only for their inciting proinflammatory condition. Thirty-three patients in the etoposide group (72%) and 32 in the no-etoposide group (67%) died during follow-up. Median survival in the etoposide and no-etoposide groups was 1.04 and 1.39 months, respectively. There was no significant difference in survival between the etoposide and no-etoposide groups (log-rank p = 0.4146). On multivariable analysis, there was no association between treatment with etoposide and survival (HR for death with etoposide = 1.067, 95% CI: 0.633–1.799, p = 0.8084). Use of etoposide-based therapy was not associated with improvement in outcomes in this large cohort of adult secondary HLH patients.
Acta Haematologica – Karger
Published: Sep 1, 2021
Keywords: Hemophagocytic lymphohistiocytosis; Adult; Secondary treatment; Etoposide
Read and print from thousands of top scholarly journals.
Already have an account? Log in
Bookmark this article. You can see your Bookmarks on your DeepDyve Library.
To save an article, log in first, or sign up for a DeepDyve account if you don’t already have one.
Copy and paste the desired citation format or use the link below to download a file formatted for EndNote
Access the full text.
Sign up today, get DeepDyve free for 14 days.
All DeepDyve websites use cookies to improve your online experience. They were placed on your computer when you launched this website. You can change your cookie settings through your browser.